Article
PLP/DM20 expression and turnover in a transgenic mouse model of Pelizaeus-Merzbacher disease.
Glia - 1 Nov 2010
Karim Saadia A, Barrie Jennifer A, McCulloch Mailis C, Montague Paul, Edgar Julia M, Iden Debrah L, Anderson Thomas J, Nave Klaus-Armin, Griffiths Ian R, McLaughlin Mark
Abstract excerpt
The most common cause of Pelizaeus-Merzbacher (PMD) is due to duplication of the PLP1 gene but it is unclear how increased gene dosage affects PLP turnover and causes dysmyelination. We have studied the dynamics of PLP/DM20 in a transgenic mouse model of PMD with increased gene dosage of the proteolipid protein gene (Plp1). The turnover of PLP/DM20 were investigated using an ex-vivo brain slice system and...
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